Management of critical valvular outflow obstruction in neonates.

Abstract

OBJECTIVE: To present a case of metastatic esthesioneuroblastoma (ENB) involving the spine. BACKGROUND: Esthesioneuroblastoma (also known as olfactory neuroblastoma) is a rare malignancy of the olfactory epithelium. Although local invasion beyond the nasal cavity and adjacent sinuses is common, distant metastases are extraordinarily rare. To date, fewer than thirty documented cases involving the spine, spinal cord, or leptomeninges have been reported. DEISGN/METHODS: Case report. Review of clinical notes, pathology, and imaging studies. RESULTS: A 67 year-old woman presented with recurrent epistaxis. Computer tomography (CT) revealed an expansile right nasal mass causing remodeling of the nasal cavity and medial orbital wall. She underwent a gross total resection and was diagnosed with ENB - Kadish Stage C (extension beyond the nasal cavity and paranasal sinuses). Post-operatively, she was treated with 59.4 Gy of radiation delivered over 33 fractions. Post treatment surveillance consisted of frequent nasal endoscopies. Twenty months after diagnosis she presented with complaints of left shoulder pain as well as lower back pain affecting her ability to walk. Neurological examination was limited by discomfort but was notable for hyperreflexia at the knees and decreased rectal tone. X-ray revealed a pathological fracture of the left humerus. Magnetic resonance imaging (MRI) of the spine showed destructive lesions at T12 and L1 with prominent extraosseous extension into the epidural and paraspinal spaces resulting in severe spinal canal stenosis, neural foraminal effacement and cord compression. CT-guided biopsy provided the diagnosis of metastatic ENB. The patient underwent a multilevel posterior laminectomy and fusion to decompress the spine. She had a full neurological recovery. CONCLUSIONS: Distant metastases from ENB are rare, but can occur despite careful surveillance for local recurrence. Patients with ENB who present with back pain should be imaged early to investigate the possibility of drop metastases. <b>Disclosure:</b> Dr. Armand has nothing to disclose. Dr. Welch has nothing to disclose.